A 64-year-old man with congenital factor V deficiency and hereditary spherocytosis was attending our hospital for type II diabetes and stage 4 diabetic nephropathy. Coronary angiography performed to assess chest pain revealed severe triple-vessel disease, including total occlusion of the right coronary artery. The patient required surgical coronary revascularization. In the preoperative examination, the activated partial thromboplastin time (APTT) and prothrombin time-international normalized ratio (PT-INR) were high (89.5 s and 1.95) and factor V activity was low (6% ; normal range, 70-135%). Hemodialysis was performed on the day of the operation, and 6 units of fresh frozen plasma (FFP) were administered, which reduced immediately the preoperative PT-INR to 1.33. We performed off-pump coronary artery bypass grafting (OPCAB) and perioperatively administered 6 units of FFP with 4 units of red blood cells (RBC) transfusion. The postoperative course of the patient was uneventful, and he was discharged on postoperative day 22. Here we report the case of a patient with a very rare disease of congenital factor V deficiency and hereditary spherocytosis complicated with stage 4 diabetic nephropathy who required OPCAB.